AB0702 Coexistence of systemic sclerosis and microscopic polyangitis associated with pulmonary renal syndrome: a case report and literature review
Authors: Y. Jiang, Y. X. He, X. Liao, S. B. Wang, Y. F. Qing
Journal: Annals of the Rheumatic Diseases (2022)

Background Systemic sclerosis is a chronic immune disease characterized by varying degrees of fibrosis of skin and internal organs. Microscopic polyangitis, as a subtype of ANCA associated vasculitis, mainly involves small blood vessels, often manifested as necrotizing glomerulonephritis and pulmonary capillary vasculitis. Pulmonary renal syndrome is characterized by diffuse alveolar hemorrhage based on pulmonary capillary vasculitis and rapidly progressive glomerulonephritis, which can be derived from a variety of autoimmune diseases, of which ANCA associated vasculitis accounts for about 60%[1]. The cases of coexistence of systemic sclerosis and microscopic polyangitis associated with pulmonary renal syndrome in clinic are rare, which is often dangerous and is easy to miss diagnosis or misdiagnosis. Objectives To investigate the clinical characteristics, diagnosis and treatment of coexistence of systemic sclerosis (SSC) and microscopic polyangitis（MPA）associated with pulmonary renal syndrome (PRS). Methods The clinical data, diagnosis and treatment process of a patient who has SSC combined with MPA and PRS were summarized and analyzed. And the literature was reviewed to explore the correlation of the pathogenesis and clinical experience of SSC complicated with MPA and PRS. Results The case is a middle-aged male who was diagnosed as SSc due to the hardening of the skin of both hands，Reynolds phenomenon, the anti-scl-70 antibody are positive. The patient suffered from repeated hemoptysis, progressive dyspnea, severe anemia and renal insufficiency, so he was diagnosed as MPA with PRS. After giving glucocorticoid, immunosuppressant and anti-infection treatment, his condition has improved. A total of 7 case reports were retrieved by reviewing the relevant literature.A total of 7 patients were reported.They were first diagnosed as SSc and then MPA with PRS, of which 4 cases improved after treatment and 3 cases died. Among the dead patients, 1 case was treated with penicillamine for 3 years, and the remaining 2 cases were only treated with steroids without immunosuppressants.In SSc, P-ANCA is closely related to vasculitis, and the prognosis of PRS secondary to P-ANCA may be very poor. Most of the diagnosis of MPA is only after patients have kidney or lung diseases.There is a certain correlation between them in pathogenesis. Glucocorticoids, immunosuppressants, biological agents, hemodialysis and plasma exchange are the main treatments. Conclusion Although the cases of SSc combined with MPA and PRS are rare, there are still many cases reported,which reminds us: ①When SSc patients have new symptoms such as renal insufficiency or lungs, they should be alert to new entities that may be combined with other autoimmune diseases to avoid missed diagnosis or misdiagnosis.②ANCA should be detected in SSc patients at baseline, which may be related to disease activity.③PRS has rapid progress and high mortality, whcih is an emergency that needs urgent treatment. Such patients should be treated with glucocorticoid, immunosuppression and plasma exchange immediately. However, if patients are complicated with SSc, they need to be extra careful when using high-dose steroids, which increases the risk of renal crisis. References [1]de Groot K, Schnabel A. Das pulmorenale Syndrom [Pulmonary-renal syndrome]. Internist (Berl). 2005;46(7):769-782. doi:10.1007/s00108-005-1423-8. Figure 1. The contrast of chest CT before and after treatment showed that the exudative lesions of both lungs were significantly absorbed Figure 2. Clinical data of systemic sclerosis combined with microscopic polyangitis and pulmonary renal syndrome Acknowledgements Institute of Research Center of Gout and Hyperuricemia of the Affiliated Hospital, North Sichuan Medical College Disclosure of Interests None declared